One decade distinct features, morbidity and mortality of scleroderma: a cross-sectional study

Conducting an epidemiologic study on scleroderma patients referred to hospitals and tertiary centres of rheumatologic diseases in Shiraz, located in south of Iran.

A cross-sectional study was done on patients' records registered in scleroderma outpatient clinics as well as hospitals associated with Shiraz University of Medical Sciences. Gathering data in pre-formed data sheets, descriptive analysis plus qualitative comparisons by chi-square test were done using SPSS 15.

In 533 medical records, female to male ratio was 7.3:1. The disease is mostly seen in 3rd and 4th decades of life. More patients had negative family histories (56.1%). 37.5% of the patients had diffuse form of the disease, 36.8% had limited one, and 17.3% had overlap syndrome, mostly, by lupus erythematosus (33%). Most common first presentation was Raynaud phenomenon (40.7%). Two most prevalent clinical manifestations were skin thickening (97.2%) and gastrointestinal involvement (68.9%). Clinical presentations were compared between three most common types of the disease plus various stages of life. Among recorded capillaroscopies, active form was the most prevalent one (38.3%). In documented serologic markers, the most common positive one was anti-nuclear antibody (ANA) (75.6%). Two most common etiologies of hospitalisation were digital ulcer (30.9%) and pulmonary fibrosis (5.7%). The most common cause of death (17) was pulmonary fibrosis (35.2%).

This study is the first epidemiologic survey on Iranian scleroderma patients with significantly large sample size compared to previous studies worldwide. It can thus provide some guidance for further multi-provincial, multinational and interracial studies on scleroderma.